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Lymphatic Malformations (LMs) are benign congenital malformations of the lymphatic system that commonly involve the abdomen in children (mesentery of the small intestine and omentum). The management of these malformations is not unique. 7 children with different ages (range: newborn to 14 years), diagnosis was incidental in some cases, while in others for abdominal pain. All patients underwent abdominal ultrasound and Magnetic Resonance Imaging (MRI). Laparoscopy for diagnosis was useful in 4 cases. Treatment was: conservative in 1 child, laparoscopic excision in 3 patients, laparotomic excision in 3 cases. At follow up we observed recurrence in a case that required integrated treatment and bowel occlusion after excision in 1 case. This benign malformations may not cause any symptoms to patient. The goal of treatment is to maintain organ function, preserve aesthetic integrity and complications control. Management of these patients could be varied: the best approach should be evaluated on the basis of the patient.

Abstract Eosinophilic gastrointestinal disorders (EGIDs) are chronic/remittent inflammatory diseases associated with a substantial diagnostic delay, often attributable to misdiagnosis and variable clinical presentation in adults. In the pediatric population, few studies have been conducted worldwide reporting EGID diagnostic delay and its consequences on patients. This study aims to analyze and identify potential clinical factors and complications associated with a longer diagnostic time. We performed a retrospective analysis of pediatric patients with EGIDs followed at the Center for Pediatric EGIDs in Pavia, Italy. A total of 60 patients with EGIDs were enrolled. Thirty-nine (65%) patients had EoE, and 21 (35%) non-esophageal EGIDs. EGID diagnosis was achieved about 2 years after the symptom onset, and the median diagnostic time was 12 months (IQR 12–24 months). Diagnostic time was 12 months (IQR 12–69) in non-esophageal EGIDs and 12 months (IQR 4–24 months) in EoE patients. EoE patients presenting with FTT and feeding issues experienced a longer diagnostic time (p = 0.02 and p = 0.05, respectively) than children without growth and feeding impairments. In this study, symptoms appeared about 2 years before the definitive EGID diagnosis was reached, and this diagnostic time was shorter than the delay observed in other published studies. Especially in EoE children, the diagnostic time is significantly associated with impaired child growth, highlighting the importance of an early diagnosis to prevent esophageal stenosis and failure to thrive.

Background: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF. Methods: Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010–2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR). Results: The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day–18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%. Conclusions: H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

Abstract Background The minimally invasive surgery (MIS) in term of robot-assisted surgery changed in a dramatic way the surgical approach either in adults or children. For many specialties (urology, gynecology, general surgery) robotic surgery rapidly became the gold standard for some procedures, while the experience in pediatric population is not wide for some reasons. The aim of this study is to retrospective analyze trends of application of robotic surgery in pediatric patients across the country, focusing on indications, limitations, development, and training acquired by national experience and in comparison to the literature. Methods We made a retrospective multicenter study on behalf of Italian Society of Pediatric Surgery. We performed a census among all pediatric surgery units in the country to enroll those performing robotic surgery on children between 2013 and 2019. Results We enrolled 7 pediatric surgery referral Centers (Ancona, Bologna, Brescia, Genova, Pavia, Pescara, Siena). A total of 303 patients were included in the study, 164 males (54%) and 139 females (46%). The most commonly performed interventions for each anatomic area were respectively atypical pulmonary resection (38%), pyeloplasty (49%), and fundoplication (30%). Conclusions Since its first application in Italy, about 10 years ago, several considerations were made about application and feasibility of robotics in children.

Pleuropulmonary blastoma (PPB) is a rare primitive malignant lung cancer that occurs in pediatric age. Its main differential diagnosis is congenital cystic pulmonary malformation (CPAM).A 30-day-old infant with respiratory failure obtained a chest x-ray and a computed tomography scan (CT) which revealed hypertensive pneumothorax with multifocal bilateral cysts. After thoracic drainage, the patient underwent multiple thoracoscopic pulmonary resections. The first histological diagnosis was of type 2 CPAM.During the radiological follow-up, an increase in the number and dimension of the lesions was detected. Thus, a histological revision was performed, leading to the diagnosis of type I PPB, at nine months. The patient subsequently underwent chemotherapy.At the five-year follow-up appointment, chest magnetic resonance (MR) and CT scans showed a dimensional increase in size of the lesions, with the risk of recurrent pneumothorax. An upper right lobectomy and wedge resection of the residual cysts were performed.Control MR scans showed normalization of the lung parenchyma and the patient showed substantial clinical improvement.

Abstract Introduction This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy. Methods A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units. Results The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern

AimThe use of robotics-assisted surgery in oncology has been proved effective and safe in adults. Despite these results, the use of robotics has been rarely reported for pediatric oncology. Our review aims to evaluate the safety and feasibility of robotics-assisted surgery in this field, analyzing our experience and performing a systematic review of the most recent studies.MethodsWe reviewed all patients affected by an oncological disease who underwent a robotics-assisted procedure at our institute. We performed a systematic review of the literature from 2012 to 2021 on the subjects.FindingsA total of 14 patients underwent robotics-assisted tumor resection. Eleven procedures (median age 13.2-years old) were carried out in children with adnexal lesions (seven tumor excision and four ovariectomies). Histological diagnosis was mature teratoma (six), serous papillary cystadenofibromas of the fallopian tube (two), ovarian serous cystadenoma (one), ovarian mucinous cystadenoma (one), and ovarian seromucinous cystadenoma. The median length of stay was 2 days. No recurrences or complications at a median follow-up of 2.1-years were observed. A 5-year-old girl underwent a complete posterior resection of a type 3 sacrococcygeal tumor with a robotics-assisted approach for the dissection of a possible intraabdominal residual component of the lesion. No intra- and postoperative complications were recorded. Complete excision of a recurrent differentiating neuroblastoma of the left para-renal region was performed on a 9-year-old girl. An idiopathic anaphylactic shock occurred 1 day after the procedure. At 9 months' follow-up, no local recurrences of the lesion were observed. Overall, we reported no conversion to open surgery. Lastly, a robotic excision of a growing left superior mediastinal intermixed ganglioneuroblastoma was performed on an 8-year-old girl with no postoperative complications. Follow-up was uneventful (7 months). In the literature, the rate of complications ranges from 0 to 28%, mainly related to difficult dissection and impaired anatomy. Conversion is reported in 5% of all oncological procedures, due to more invading tumors and altered anatomical features. No robotics-related complications were reported.ConclusionRobotics-assisted surgery in pediatric oncology has proven to be feasible. Nevertheless, its use should be limited to selected cases and performed by highly trained oncological surgeons. Preparation and patient positioning, alongside a correct port placement, are crucial to carrying out these procedures. Further innovations in robotics may allow a wider application of this technology in pediatric oncology.

Congenital lung malformations (CLMs) involve anomalies of the lungs and respiratory tree such as congenital pulmonary airway malformation (CPAM), pulmonary sequestration (PS), bronchogenic cysts, congenital lobar emphysema, and bronchial atresia (BA). Although symptomatic lesions require surgical resection, the appropriateness of surgery for patients with asymptomatic malformations is a matter of ongoing debate. Limited data are available concerning the long-term follow-up of affected subjects. In this study, we sought to evaluate the long-term clinical and functional respiratory outcomes in children with CLMs who underwent surgical resection. We carried out a retrospective analysis of 77 children with CLMs who underwent pulmonary resection with at least one year of follow-up. The most common diagnoses were CPAM (50.65%), hybrid lesions (25.97%), lobar emphysema (11.69%), and PS (5.19%). The most common surgical approaches were lobectomy (61.3%), segmentectomy (10.7%), and pneumonectomy (5.3%). Acute post-surgery complications occurred in 31.2% of children. In addition, 73.7% experienced long-term complications, and we found no correlation between the presence of these complications and the sex of the patients, their age at time of surgery, the type of surgery undergone, the presence of symptoms prior to intervention, or acute complications after surgery. Pulmonary function tests revealed FEV1 Z-scores of p = 0.031). In conclusion, clinical and functional respiratory complications may occur in children with CLMs who undergo surgical resection. Long-term monitoring is needed to improve the management of asymptomatic patients.

Aim: Video-assisted thoracoscopic surgery (VATS) has been widely used in the last decades. Nevertheless, the pros and cons of thoracoscopy vs. open surgery in pediatric oncology are still under debate. In literature, VATS has been applied for both diagnostic and ablative surgery to treat neurogenic tumors, thymic neoplasms, lung tumors and metastases, germ cell tumors, lymphoproliferative diseases, and other rare tumors. Recent reviews described excellent outcomes in pediatric oncology as well as in the treatment of adult lung cancer, with a significantly higher rate of mortality and complication in thoracotomy compared to VATS. We reviewed our experience on thoracoscopy in pediatric malignancy and compared it to the literature.Materials and Methods: This was a retrospective cohort-study of pediatric oncological patients who underwent VATS at our institution from 2007 to 2020, and a review of the recent literature on the topic.Results: A total of 43 procedures were performed on 38 oncological patients (18 males, 20 females). Median age was years 7.72 (0.35–18.6). Diagnosis: 10 neurogenic tumors, nine hematological diseases, five metastases, four lypoblastomas, three thymic pathologies, three germ cell tumors, two pleuropneumoblastomas, two myofibroblastic tumors, one myoepithelial carcinoma, one liposarcoma, and three suspected oncological mass. In three cases, a 3D model was elaborated to better plan the surgical approach. Diagnostic biopsies were 22 (51.1%), and ablative surgeries, 21 (48.9%). One neurogenic tumor was resected with the Da Vinci Robot. Median operative time was 120 min (30–420). A drain was left in place in 20 (46.5%) for a median of 4 days. Median length of hospitalization was 5 days (1–18). One case (2.3%) was converted (intraoperative bleeding). There were three post-operative complications (7.0%): one pneumonia, one pleural effusion, and one diaphragmatic paralysis (need for plication). Results were compared to recent literature, and morbidity and conversion rate were comparable to reviewed publications.Conclusion: VATS represents a valuable tool for diagnostic and therapeutic procedures in pediatric oncology. Nonetheless, it is a challenging technique that should be performed by expert surgeons on oncological and mini-invasive surgery. Three-dimensional reconstruction can optimize the pre-operative planning and guarantee a safer and more targeted treatment. Finally, the advent of robotics-assisted surgery represents a new challenge that may further implement the advantages of VATS.

Primary Obstructive Megaureter (POM) is a common cause of hydronephrosis in children with spontaneous resolution in most cases. High-Pressure Balloon Dilatation (HPBD) has been proposed as a minimally invasive procedure for POM correction in selected patients. The aim of the paper is to review our experience with HPBD in patients with POM. We performed a retrospective study in a single Centre collecting data on patients’ demographics, diagnostic modalities, surgical details, results and follow-up. In particular, the endoscopic aspect of the orifice permitted the identification of 3 patterns: adynamic ureteral segment, stenotic ureteric ring and pseudoureterocelic orifice. We performed HPBD in 30 patients over 6 years. We had 23 patients with adynamic distal ureteral segment (type 1), 4 with stenotic ring (type 2) and 3 with ureterocelic orifice (type 3). In 3 patients (10%) the guidewire did not easily pass into the ureter requiring ureteral stenting or papillotomy. Post-operative course was uneventful. Five patients (3 pseudoureterocelic) required open surgery during follow-up. HPBD for the treatment of POM is a safe and feasible procedure and it can be a definitive treatment of POM. Complications are mainly due to double J stent and none of our patients had symptoms related to vescico-ureteral reflux. The aspect of the orifice, identified during cystoscopy, seems to correlate with the efficacy of the dilatation: type 1 and 2 are associated with good and excellent results respectively; type 3 do not permit dilatation in almost all cases requiring papillotomy. HPBD can be performed in selected patients of all paediatric ages as first therapeutic line. The presence of a pseudoureterocelic orifice or long stenosis might interfere with the ureteral stenting and seems associated with worse outcomes.

Background: Over the last 30 years, the incidence of pediatric urolithiasis (PU) has been increasing and the surgical management has evolved toward a minimally invasive approach (MIA). We reported the experience of two Centers of Pediatric Surgery in the management of PU, focusing on MIA as first choice in treatment.Methods: Data were retrospectively analyzed from October 2009 to October 2019 in children with urolithiasis who were admitted to two referral Italian Centers of Pediatric Surgery. Demographic and clinical data of the patients, features of the urolithiasis, type of surgery were considered.Results: Seventy patients (7.3 ± 5.0 years) with normal renal function were treated for calculi in the pyelocaliceal system (45.7%), ureter (34.3%), bladder (4.3%), urethra (1.4%), and multiple locations (14.3%). Size of calculi was >10 mm in 55.7% of cases (kidney>bladder/urethra>multiple>ureter, p = 0.01). Symptoms were present in 75.7% of patients. Family history was positive in 16.9% of cases. MIA was performed in 59 patients (84.3%): 11.8% shockwave lithotripsy (kidney>ureter>multiple); 32.2% ureteral retrograde surgery (ureteral>other localizations); 30.5% retrograde intrarenal surgery (kidney>other localizations); and 25.4% other procedures including percutaneous nephrolithotomy, cystoscopic bladder stone removal or laser cystolithotripsy (kidney>bladder>multiple). Preoperative stenting was necessary in 52.8% of cases. Four MIA procedures (6.9%, kidney>ureter/multiple) were converted to open surgery. Open surgery was required as first approach in 15.7% of patients (kidney>ureter>multiple) who needed urgent surgery or had associated congenital renal anomalies. In 18/70 of children (25.7%), with prevalence of stones in kidney and multiple location (p < 0.01), a second procedure completed the treatment (88.8% MIA). Intraoperative difficulties were recorded in 8.5% of cases, without difference between location and size of calculi. Late complications (5.7%) were related to displacement and infection of the ureteral stent.Conclusions: MIA resulted to be feasible in more than 75% of primary surgery and in more than 85% of cases requiring a second procedure. Preoperative stent was mandatory in more than 50% of children. The technological evolution allowed to overcome many of the technical difficulties related to the approach to the papilla and lower calyxes. Open surgery is reserved for selected cases and endoscopic surgery represents the best choice of treatment for PU.

Introduction: Gastrointestinal infantile hemangiomas (IHs) are extremely rare and represent a diagnostic challenge, especially in newborns for the limited applicability of diagnostic approaches.We report two cases of neonates with gastrointestinal bleeding from intestinal hemangioma and a literature review of gastrointestinal IHs. Case 1: Full-term baby, asymptomatic at birth. On the seventh day of life she experienced failure to thrive, weight loss and hypotonus followed by melena and vomit. The imaging showed an intestinal vascular anomaly. We performed exploratory laparotomy and intestinal resection to remove the lesion. No complication occurred. Case 2: A 20-day old girl had melena and severe anaemia. The scintigraphic evaluation excluded gastric heterotopia. We opt for explorative surgery and we performed a laparoscopy that showed a congested and oedematous intestinal segment that was resected. Post-operative course was uneventful. Discussion: Gastrointestinal IHs may cause intractable bleeding, intussusception and intestinal perforations with bad prognosis. A prompt and accurate diagnosis is vital but not always easy. Colour-coded Duplex Scan are a good first-line imaging, MRI and CT give a better definition but the latter exposes to radiation. In the “era” of minimally invasive surgery, laparoscopy can have a role in the management of patients with gastrointestinal IHs. Conclusions: Gastrointestinal IHs should be considered in the differential diagnosis of neonates with anemia and gastro-intestinal bleeding. In selected cases, the minimally invasive approach permits diagnosis and treatment. Keywords: Gastrointestinal hemangioma, Neonates, Laparoscopy, Vascular anomalies, Gastrointestinal bleeding

Introduction: Situs ambiguous (SA) is a rare clinical entity that includes a spectrum of abnormalities in which organs and vessels are opposed to their normal arrangement. SA is frequently associated with other malformations such as splenic alterations, biliary atresia and vascular abnormalities. We report two cases of patients with SA, polysplenia and choledochal cyst. This association has never been described to date. Case reports: Two patients, both males, came to our attention at the ages of 7 and 8 months after ultrasound identification of choledochal cysts. One patient, who had a prenatal suspicion of situs ambiguous and dextrocardia, presented with failure to thrive and increased hepatic enzymes. The other patient was asymptomatic, but prenatal evaluation showed gastric malposition. Post-natal laboratory tests were normal. Preoperative radiological diagnostic work-up showed polysplenia, intestinal anomaly of rotation and fixation, pre-duodenal portal vein in one baby. Both patients underwent surgical correction by cyst excision and Roux-en-Y hepaticojejunostomy. Follow-up (3 months and 3.5 years respectively) was uneventful. Biochemical hepatic parameters normalized soon after surgery. Discussion: Situs anomalies in children are often associated with other malformations. The most common are splenic and cardiac defects but intestinal malformations and hepatico-biliary abnormalities have been reported too. Above all, the BASM (biliary atresia splenic malformation) syndrome has been extensively examined, obtaining important information on the etiology of the cystic form of biliary atresia. Conclusions: A careful evaluation of patients with situs ambiguous is important to exclude associated anomalies and to plan the most appropriate surgical approach. The association with choledochal cyst should be taken into account. Keywords: Choledochal cyst, Situs ambiguous, Polysplenia

Aim of the study: We would like to report an unusual case of mature cystic teratoma (MCT) of the left colon presenting with intestinal obstruction in a syndromic patient with low-anorectal malformation (ARM). Case report: The boy had multiple malformations: low ARM with perineal fistula, situs ambiguous, dextrocardia, double outlet right ventricle with pulmonary stenosis and anterior aorta.At two years he required cardiac surgery but he developed intestinal obstruction. The intestinal condition put us in a diagnostic challenge: we thought about intestinal stenosis with fecal impaction but radiological investigations were inconclusive. During surgery we identified an abdominal mass that was resected together with the adjacent descending colon. Histology showed MCT. Follow-up was uneventful. Conclusions: MCT rarely occurs outside the sacrococcygeal–gonadal location. They are frequently associated with ARM in Currarino Syndrome: the mass is located in front of the sacrum and causes anal stenosis. Nevertheless, in the reported patient, MCT did not arise from the spine but from the left colon.Paediatric cases of colonic MCT have never been reported. Affected adults can develop constipation and/or intestinal obstruction. Surgery permits to remove the mass in both symptomatic and asymptomatic patients. Keywords: Mature cystic teratoma, Dermoid cyst, Anorectal malformation, Intestinal obstruction, Paediatric surgery

Abstract Spinal muscular atrophy (SMA) includes genetic heterogeneous diseases characterized by muscle weakness and atrophy. There are at least two risk factors for SMA patients to develop urinary dysfunctions: 1) the neural degeneration, that influences the pelvic floor and sphincters; 2) the disabilities that predispose to constipation and urinary incontinence and symptoms. Nevertheless, the true incidence of urological disorders remains unknown. The evaluation of urinary symptoms in SMA patients remains scarcely investigated. The aim of our paper is to assess the association between urological disorders and SMA. We worked out a questionnaire to assess the voiding habits of patients with SMA followed at a single Italian Centre. We collected demographic data and information regarding respiratory, orthopedic and gastrointestinal involvement. We excluded patients

Primary myoepithelial carcinoma of the lung (PMC-L) arising from the bronchial glands in lower respiratory tract is exceedingly rare. Thus far, few cases in adults and only one in a pediatric patient have been recorded. To our knowledge, this is the first report of PMC-L successfully removed in a child, focusing on the importance of multidisciplinary primary surgery for the treatment of this tumor. A 7-year-old girl was admitted for persistent cough and fever; she was unresponsive to oral antibiotics. Chest radiography showed loss of volume of left lung sustained by almost total atelectasis. After routine clinical investigations, she was referred for computed tomography scan and magnetic resonance imaging that documented the presence of a mass occupying the entire left upper lobe, infiltrating the pulmonary hilum (main bronchus, pulmonary artery, superior pulmonary vein, and pericardium). After multidisciplinary evaluation, the histopathologic diagnosis of PMC-L was established using ultrasonography-guided transthoracic core needle biopsy and bronchoscopic biopsies. She was then subjected to left pneumonectomy under extracorporeal circulation and positioning of a thoracic expander filled with 200 mL of saline solution. The postoperative course was uneventful. With TREP (very Rare Tumor in Pediatric Age) consent radiotherapy was performed (61.2 Gy). At the 10-month follow-up, the patient was alive, breathing normally without any oxygen support, without recurrence of PMC-L or metastasis, and without any chest deformity. To our knowledge, this is the first case where a pediatric patient was successfully operated for PMC-L involving the whole lung. Extracorporeal circulation enabled us to perform radical primary surgery. Prosthesis implant not only maintained normal chest expansion but also allowed focused radiotherapy, thus enabling us to prevent damage to vital organs.

Aim: To report the case of a patient with total colonic tubular duplication pointing out the diagnostic and therapeutic difficulties and reviewing the Literature concerning this rare malformation. Case report: The patient was a female girl born at term with vertebral and spinal anomalies. At 6 months she underwent emergent left upper lobectomy for a symptomatic, previously undiagnosed left congenital lobar emphysema. She also had intractable coprostasis and abdominal distension. The clinical picture and the results of the contrast enema made us think of congenital megacolon but suction rectal biopsies were inconclusive. Therefore we obtained full thickness colonic and terminal ileum biopsies and we performed an ileostomy for faecal diversion and bowel management. A few months later, the anterograde contrast study from the distal ileostomy showed two colons with proximal communication and a distal pouch. On laparotomy, we confirmed the diagnosis of complete colonic tubular duplication. We divided the common wall between the two colons, we performed a mucosectomy and we removed the duplicated segment. Post-operative course was uneventful. Conclusions: Duplications of the colon are rare and the diagnosis is not easy. Two main problems occurred during surgery: differentiating between normal and duplicated colon and removing the duplication avoiding extensive colonic resections. Resection of the common wall, mucosectomy and removal of the duplicated colon represented a feasible and safe procedure. Keywords: Colon duplication, Constipation, Paediatric surgery, Congenital malformation

Background: Long-term negative sequelae of esophageal atresia (EA) may induce poor growth and impaired nutritional status in childhood. We describe the nutritional profile and energy metabolism of children with repaired EA to identify malnutrition risk factors and optimize growth management. Methods: Twenty-one children (>4 years) were included, and anthropometric measurements, nutritional assessment, and energy metabolism were considered. The subjects were defined as undernourished if they met BMI < −2 standard deviation (SD). To grade undernutrition, we defined the prevalence of underweight, stunting, and wasting (cut-off level of p < 0.01). Wasting was noted in 28.6% of patients, of these 5 children were undernourished (p < 0.001) and stunting was noticed in only one patient with malnutrition (p = 0.5). Resting expenditure energy (REE) was lower in undernourished subjects compared to subjects with adequate nutritional status (p < 0.001). Malnutrition was associated to: type of EA (p = 0.003, particularly type A and C); intervention including deferred anastomosis due to long-gap repair (p = 0.04) with/or without jejunostomy (p = 0.02), gastric pull-up (p = 0.04), primary anastomosis (p = 0.04), pyloromyotomy in long-gap (p < 0.01); small for gestational age condition (p = 0.001). Conclusions: undernutrition risk factors, beyond the type of malformation, surgery, and perinatal factors, must be early considered to personalize nutritional programming. Energy metabolism is important to monitor the nutritional requirements. The management of nutritional issues is surely a contributory factor able to counteract the poor growth of children with EA.

Abstract Microgastria is a rare congenital condition often associated with other anomalies. In the present report we describe the case of a 6-year-old girl with isolated CM who presented with dumping syndrome successfully treated by a Hunt-Lawrence pouch.